Epstein-barr virus-associated lymphoepithelioma-like carcinoma of mandible

We describe the first case of an lymphoepithelioma-like carcinoma (LEC) of the mandible with a view to discuss probable histogenesis from salivary gland inclusions in the mandible, effective therapy, the unusual pattern of nodal metastases, and association with the Epstein-Barr virus. An 11-year-old boy presented with a lytic lesion in the left mandible and bilateral neck node enlargement. Histologically, the biopsied metastatic neck nodes showed sinusoids distended with histiocytes and single large atypical cells that mimicked Hodgkin lymphoma. The mandibular tumor on biopsy showed morphology similar to an undifferentiated carcinoma or LEC of nasopharynx with nuclear Epstein-Barr virus-associated in situ hybridization signals. The patient received ifosfamide, cisplatin, and etoposide chemotherapy followed by a hemimandibulectomy, which did not reveal any residual tumor. Postoperative radiotherapy was completed, and 3 years later the patient is alive and free of disease.